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Blood, 15 November 2008, Vol. 112, No. 10, pp. 4009-4016. Prepublished online as a Blood First Edition Paper on August 15, 2008; DOI 10.1182/blood-2008-02-138156.
Submitted February 11, 2008
Department of Medicine, Royal Free and UCL Medical School, London, United Kingdom * Corresponding author; email: a.wechalekar{at}medsch.ucl.ac.uk.
AL amyloidosis associated with IgM paraproteinemia is rare. We report the largest series to date of 103 consecutive such patients evaluated at the UK National Amyloidosis Centre between 1988- 2006. Renal, cardiac and lymph node amyloid was present in 53%, 35% and 21% of patients at presentation and
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| Copyright © 2008 by American Society of Hematology Online ISSN: 1528-0020 | |||||||||
2 organ were involved in 54%. 73% were reported to have an abnormal bone marrow infiltrate (lymphoid in 87%). The median IgM paraprotein was 8g/L and serum free light chain (FLC) ratio was abnormal in 77/87 (88%). The abnormal FLC component was >100mg/L in only 31% cases. 32% achieved a partial haematologic response to treatment with no complete responders and there appeared to be a greater response to combination regimes than single agent oral alkylators (59% versus 20% respectively, p=0.003). Four achieved amyloidotic organ responses, organ function remained stable in 68%. None with lymph node involvement showed nodal improvement. Median overall survival was 49 months. AL amyloidosis with IgM paraproteinemia represents a distinctive subset of patients with AL amyloidosis who are likely to have wider variety of underlying clonal disorders (often lymphoid) than AL in general, have low level FLC abnormality and should be treated with appropriately tailored chemotherapeutic regimes for the underlying clonal disorder.
